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Cardiology1126 papers

Sarcoma

Last edited: 4/23/2026

Overview

Sarcomas are a heterogeneous group of malignant tumors arising from connective tissues, including bone, soft tissue, and rarely, cardiac structures. They encompass various subtypes with distinct biological behaviors and prognoses.

Diagnosis

  • Clinical Presentation: Varies by location (e.g., mass, pain, constitutional symptoms) 1322.
  • Imaging: CT, MRI, PET scans for localization and staging 1232233.
  • Histopathology: Essential for definitive diagnosis; immunohistochemistry aids in subtype classification 571020253134353740414547505155576061636467687071737578808183.
  • Genetic Testing: Useful for identifying specific genetic alterations relevant to prognosis and treatment 91077.
  • Management

  • Surgical Resection: Primary treatment for localized disease; extent depends on location and feasibility 412293748556375.
  • Adjuvant Therapy: Chemotherapy (e.g., gemcitabine, doxorubicin) and radiotherapy post-surgery to improve outcomes 118283444556375.
  • Targeted Therapy: Tazemetostat for specific molecular alterations (e.g., EZH2 mutations) 1.
  • Immunotherapy: Pembrolizumab combined with talimogene laherparepvec shows promise in some cases 18.
  • Anticoagulation: Direct oral anticoagulants or low-molecular-weight heparin for VTE prophylaxis post-surgery 8.
  • Special Populations

  • Pediatrics: Limited data; tailored surgical approaches and multidisciplinary care are crucial 60.
  • Elderly: Consider functional status and comorbidities; less aggressive treatment may be warranted 55.
  • Comorbidities: Presence of comorbidities like heart disease influences treatment decisions and outcomes 233344556375.
  • Key Recommendations

  • Surgical resection remains the cornerstone of treatment for localized sarcomas 412293748556375 (Evidence: Strong).
  • Adjuvant chemotherapy and radiotherapy should be considered based on tumor subtype and stage to improve survival 118283444556375 (Evidence: Moderate).
  • Genetic testing is recommended to guide personalized treatment strategies and identify potential targeted therapies 91077 (Evidence: Moderate).
  • References

    Showing 100 most recent of 180 indexed papers.

    1 Liu Q, Luo M, Gao M, Yang B, Liu X, Liang G. A Real-World Disproportionality Analysis of FDA Adverse Event Reporting System Events for Tazemetostat. Therapeutic innovation & regulatory science 2026. link 2 Zhang X, Zhuo Y, Zhang D. Dual Left Atrial Masses Causing Inflow Obstruction: A Rare Presentation of Primary Cardiac Intimal Sarcoma. Vascular health and risk management 2026. link 3 Zeng A, South A, Slevin M, Lim K. Persistent pain and fever unmasking aortic angiosarcoma at the site of previous endovascular aortic repair. BMJ case reports 2025. link 4 Jejurikar N, Herrero C, Fabbri N. The Role of Distraction Osteogenesis in Limb Salvage for Tumors. Bulletin of the Hospital for Joint Disease (2013) 2024. link 5 Siddiqui H, Osmani AH. Spinal Myeloid Sarcoma in a Non-Leukaemic Patient. Journal of the College of Physicians and Surgeons--Pakistan : JCPSP 2024. link 6 Bergonzoni E, De Gaspari M, D'Onofrio A, Cibin G, Rizzo S, Basso C et al.. A cardiac intimal sarcoma mimicking infective endocarditis. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2024. link 7 Khan R, Sunthankar KI, Yasinzai AQK, Tareen B, Zarak MS, Khan J et al.. Primary cardiac sarcoma: demographics, genomic study correlation, and survival benefits of surgery with adjuvant therapy in U.S. population. Clinical research in cardiology : official journal of the German Cardiac Society 2024. link 8 LiBrizzi CL, Agarwal AR, Chiu AK, Morris CD, Thakkar SC, Levin AS. The efficacy and safety of direct oral anticoagulants compared with low-molecular-weight heparin for venous thromboembolism prophylaxis after surgical resection of primary lower extremity bone or soft-tissue sarcoma. Journal of surgical oncology 2024. link 9 . Heritable Defects in Mitotic and Telomere Function Confer Sarcoma Risk. Cancer discovery 2023. link 10 Mandelker D, Ladanyi M. Germline study points to sarcoma pathways. Science (New York, N.Y.) 2023. link 11 Arnold MT, Geiger EJ, Hart C, Greig D, Trikha R, Sekimura T et al.. Is High-dose Radiation Therapy Associated With Early Revision Due to Aseptic Loosening in Patients With a Sarcoma of the Lower Extremities Reconstructed With a Cemented Endoprosthesis?. Clinical orthopaedics and related research 2023. link 12 Chan EY, Ali A, Zubair MM, Nguyen DT, Ibarra-Cortez SH, Graviss EA et al.. Primary cardiac sarcomas: Treatment strategies. The Journal of thoracic and cardiovascular surgery 2023. link 13 Makarov IA, Papko KA, Makarova TA, Bendov DV, Mitrofanova LB. Heart Transplantation as a Treatment Option for Recurrent Myxofibrosarcoma: A Clinical Case. Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation 2023. link 14 Helbig D, Ziemer M, Dippel E, Erdmann M, Hillen U, Leiter U et al.. S1-guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2022. link 15 Kauzonas E, Keturakis V, Žukovas G, Jakuška P, Benetis R. A riddle yet unsolved: two case reports of primary cardiac sarcoma. Acta chirurgica Belgica 2022. link 16 Kilitci A, Yilmaz F, Kilicgun H, Uygun N. A Case of Primary Undifferentiated Pleomorphic Sarcoma of the Lung. Journal of the College of Physicians and Surgeons--Pakistan : JCPSP 2021. link 17 Yin K, Luo R, Wei Y, Wang F, Zhang Y, Karlson KJ et al.. Survival outcomes in patients with primary cardiac sarcoma in the United States. The Journal of thoracic and cardiovascular surgery 2021. link 18 . Talimogene Laherparepvec plus Pembrolizumab Is Effective in Sarcomas. Cancer discovery 2020. link 19 Sleijfer S, Lolkema M. Bridging the Translational Divide in Oncology: . Clinical cancer research : an official journal of the American Association for Cancer Research 2020. link 20 Furukawa H, Honda T, Yamasawa T, Kanaoka Y, Tanemoto K. Surgical management of large undifferentiated cardiac sarcoma involving the right ventricle to pulmonary trunk. General thoracic and cardiovascular surgery 2020. link 21 Guan T, Zhang H, Lin W, Qiu Z, Yang J, Su M et al.. Prognostic Impacts of Tumour Size on Patients With Primary Cardiac Sarcoma. The Canadian journal of cardiology 2020. link 22 Wei C, Hu B, Peng X, Li X, Zhao Y, Peng D et al.. Pericardial Sarcoma: "Invisible" on Radiology. The Canadian journal of cardiology 2020. link 23 Shamai S, Rozenbaum Z, Merimsky O, Derakhshesh M, Moshkovits Y, Arnold J et al.. Cardio-toxicity among patients with sarcoma: a cardio-oncology registry. BMC cancer 2020. link 24 Martin-Broto J, Hindi N. In Reply. The oncologist 2019. link 25 Chen TW, Loong HH, Srikanthan A, Zer A, Barua R, Butany J et al.. Primary cardiac sarcomas: A multi-national retrospective review. Cancer medicine 2019. link 26 Alcindor T, Al-Fakeeh A, Goulding K, Solymoss S, Ste-Marie N, Turcotte R. Venous Thromboembolism in Patients with Sarcoma: A Retrospective Study. The oncologist 2019. link 27 de Baere T, Tselikas L, Gravel G, Hakime A, Deschamps F, Honoré C et al.. Interventional radiology: Role in the treatment of sarcomas. European journal of cancer (Oxford, England : 1990) 2018. link 28 Vaswani D, Wallace AN, Eiswirth PS, Madaelil TP, Chang RO, Tomasian A et al.. Radiographic Local Tumor Control and Pain Palliation of Sarcoma Metastases within the Musculoskeletal System with Percutaneous Thermal Ablation. Cardiovascular and interventional radiology 2018. link 29 Kavakbasi E, Scheld HH, Kessler T, Sindermann JR, Kösek V, Tjan TDT et al.. Postoperative Complications and Long-Term Results after Primary Cardiac Sarcoma Resection. The Thoracic and cardiovascular surgeon 2018. link 30 Kosťun J, Bouda J, Vlasák P, Bartáková A, Berezovskiy D, Weinberger V et al.. Incidence of uterine sarcomas in West Bohemia, at the Department of Gynaecology and Obstetrics, University Hospital in Pilsen, Czech Republic. Ceska gynekologie 2017. link 31 Piffaretti G, Ferraro S, Carrafiello G, Macchi E, Bacuzzi A, Castelli P. Thoracic Endovascular Aortic Repair for Embolizing Total Occlusion of the Descending Aorta due to Aortic Sarcoma. Annals of vascular surgery 2017. link 32 Chaturvedi A. A Changing Force in My Life. Journal of pain & palliative care pharmacotherapy 2016. link 33 Kamran M, Fowler KJ, Mellnick VM, Sicard GA, Narra VR. Multimodality Imaging Approach towards Primary Aortic Sarcomas Arising after Endovascular Abdominal Aortic Aneurysm Repair: Case Series Report. Cardiovascular and interventional radiology 2016. link 34 Elsayad K, Lehrich P, Yppaerilae-Wolters H, Dieckmann C, Kriz J, Haverkamp U et al.. Primary Cardiac Angiosarcoma Treated With Positron Emission Tomography/Magnetic Resonance Imaging-Guided Adaptive Radiotherapy. The Canadian journal of cardiology 2016. link 35 Byon JH, Kwak HS, Chung GH, Jang KY. Acute stroke from tumor embolus in a patient with cardiac sarcoma: Aspiration thrombectomy with Penumbra catheter. Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences 2016. link 36 Stieger-Vanegas SM, Bottorff B, Sisson D, Löhr CV. IMAGING DIAGNOSIS-MULTIMODALITY FINDINGS IN AN ADULT DOG WITH PRIMARY SARCOMA OF THE PULMONARY ARTERY AND MYOCARDIAL METASTASES. Veterinary radiology & ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology Association 2016. link 37 Ramlawi B, Leja MJ, Abu Saleh WK, Al Jabbari O, Benjamin R, Ravi V et al.. Surgical Treatment of Primary Cardiac Sarcomas: Review of a Single-Institution Experience. The Annals of thoracic surgery 2016. link 38 Ohnaka M, Kyogoku M, Nakajima H, Osada H, Meshii K, Kanemitsu N. Surgical resection of two independent primary intimal sarcomas in the left atrium. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2016. link 39 Ujihira K, Yamada A, Nishioka N, Iba Y, Maruyama R, Nakanishi K et al.. A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure. Journal of cardiothoracic surgery 2016. link 40 Kholaif N, O'Neill BJ. Spindle cell sarcoma of the left atrium: an extremely rare and challenging tumour often masquerading as left atrial myxoma. The Canadian journal of cardiology 2015. link 41 Guerriero A, Giovenali P, La Starza R, Mecucci C, Montesi G, Pasquino S et al.. Metachronous cardiac and cerebral sarcomas: case report with focus on molecular findings and review of the literature. Human pathology 2015. link 42 Astarcıoğlu MA, Kalçık M, Gürsoy MO, Gündüz S, Özkan M. Multimodality imaging of a recurrent case of right-sided cardiac leiomyosarcoma with an unusual clinical course. Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir 2015. link 43 Rusthoven CG, Liu AK, Bui MM, Schefter TE, Elias AD, Lu X et al.. Sarcomas of the aorta: a systematic review and pooled analysis of published reports. Annals of vascular surgery 2014. link 44 Soo A, Molloy F, Herron B, Parissis H. Complete right ventricular obstruction caused by a giant cardiac myxoid sarcoma. Asian cardiovascular & thoracic annals 2014. link 45 Onitsuka H, Nishida T, Akashi K, Tominaga R. Total resection of the aortic arch intimal sarcoma using the L-incision technique. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2014. link 46 Park JH, Choe H, Jang WI, Hur G. Primary pericardial spindle cell sarcoma mimicking left main coronary artery disease. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2012. link 47 Takeda K, Masai T, Ishida M, Sunagawa G. Unusual presentation of multiple recurrent cardiac sarcomas as acute acalculous cholecystitis caused by rapid expansion into the right cardiac chamber: report of a case. Surgery today 2012. link 48 Locker GJ, Fuchs EM, Worel N, Bojic A, Heinrich G, Brodowicz T et al.. Whole body hyperthermia by extracorporeal circulation in spontaneously breathing sarcoma patients: hemodynamics and oxygen metabolism. The International journal of artificial organs 2011. link 49 Hales SL, Locke R, Sandison A, Jenkins M, Hamady M. Aortic angiosarcoma: a rare cause for leaking thoracic aneurysm. Cardiovascular and interventional radiology 2011. link 50 Turhan N, Ozgüler Z, Cağlı K, Cağlı K, Gölbaşı Z. Primary cardiac undifferentiated sarcoma: role of intraoperative imprint cytology and frozen section of two cases. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2011. link 51 Ye Z, Shi H, Peng T, Han A. Clinical and pathological features of high grade primary cardiac osteosarcoma. Interactive cardiovascular and thoracic surgery 2011. link 52 Patil HR, Singh D, Hajdu M. Cardiac sarcoma presenting as heart failure and diagnosed as recurrent myxoma by echocardiogram. European journal of echocardiography : the journal of the Working Group on Echocardiography of the European Society of Cardiology 2010. link 53 Tanaka M, Tabata M, Shimokawa T, Takanashi S. The rupture of descending thoracic aorta due to the necrosis of aortic intimal sarcoma. Interactive cardiovascular and thoracic surgery 2010. link 54 Chughtai B, Samadi D, Guru K, Frischer Z, Rehman J. Granulocytic sarcoma of the adrenal gland. The Canadian journal of urology 2009. link 55 Truong PT, Jones SO, Martens B, Alexander C, Paquette M, Joe H et al.. Treatment and outcomes in adult patients with primary cardiac sarcoma: the British Columbia Cancer Agency experience. Annals of surgical oncology 2009. link 56 Jiang WX, Gao CR, Sun JH, Bunai Y. Sudden cardiac death caused by a primary intimal sarcoma of the left coronary artery. International journal of legal medicine 2009. link 57 Kong PK, Theodorou S, Beran E, Singh H, Dimitri W. Primary cardiac undifferentiated sarcoma of the right atrium presenting with cardiac tamponade. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2009. link 58 Lee SH, Kim WH, Choi JB, Lee SR, Rhee KS, Chae JK et al.. Huge primary pleomorphic leiomyosarcoma in the right ventricle with impending obstruction of both inflow and outflow tracts. Circulation journal : official journal of the Japanese Circulation Society 2009. link 59 Pollock RE. The seminal role of Cancer in our understanding of sarcoma. Cancer 2008. link 60 Eisenstat J, Gilson T, Reimann J, Sampson B. Low-grade myofibroblastic sarcoma of the heart causing sudden death. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2008. link 61 Luo J, Melnick S, Rossi A, Burke RP, Pfeifer JD, Dehner LP. Primary cardiac alveolar soft part sarcoma. A report of the first observed case with molecular diagnostics corroboration. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 2008. link 62 Jerbi S, Romdhani N, Tarmiz A, Mlika S, Nizar K, Beltaïfa M et al.. Primitive cardiac sarcomas: about 2 cases. La Tunisie medicale 2007. link 63 Afonso PV, Antunes MJ. Primary cardiac angiosarcoma. Extended resection of the right atrial wall. Case report. Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology 2007. link 64 Maki RG. Future directions for immunotherapeutic intervention against sarcomas. Current opinion in oncology 2006. link 65 Lee CH, Chao TH, Tsai LM, Lin PY, Tsai WC, Yan JJ et al.. Transesophageal echocardiographic recognition of infiltrative cardiac sarcoma mimicking mitral stenosis. The international journal of cardiovascular imaging 2006. link 66 Jardin F, Vasse M, Debled M, Dominique S, Courville P, Callonnec F et al.. Intense paraneoplastic neutrophilic leukemoid reaction related to a G-CSF-secreting lung sarcoma. American journal of hematology 2005. link 67 Miller DV, Deb A, Edwards WD, Zehr KJ, Oliveira AM. Primary synovial sarcoma of the mitral valve. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2005. link 68 Kappler M, Bache M, Bartel F, Kotzsch M, Panian M, Würl P et al.. Knockdown of survivin expression by small interfering RNA reduces the clonogenic survival of human sarcoma cell lines independently of p53. Cancer gene therapy 2004. link 69 Myruski KS, Manecke GR, Kotzur A, Wahrenbrock EA, Jamieson SW. Late recurrence of cardiac sarcoma presenting as giant pulmonary artery aneurysm. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2004. link 70 Bloomberg RD, Butany JW, Cusimano RJ, Leask RL. Primary cardiac sarcoma involving the pulmonary artery and valve. The Canadian journal of cardiology 2003. link 71 Blazer DG, Sabel MS, Sondak VK. Is there a role for sentinel lymph node biopsy in the management of sarcoma?. Surgical oncology 2003. link00030-6) 72 D'Andrea N, Romano V, Mattioli F, Candia S, Montanari A, Guidi L et al.. Pulmonary artery leiomyosarcoma with thyroid metastases. Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace 2003. link 73 Gupta ML, Srivastava CP, Bageratta M, Katara R. Pulmonary leiomyosarcoma. The Indian journal of chest diseases & allied sciences 2002. link 74 Samal AK, Ventura HO, Berman A, Okereke C, Gilliland YE, Willis GW. Myxosarcoma: a rare primary cardiac tumor. The Journal of the Louisiana State Medical Society : official organ of the Louisiana State Medical Society 2002. link 75 Talbot SM, Taub RN, Keohan ML, Edwards N, Galantowicz ME, Schulman LL. Combined heart and lung transplantation for unresectable primary cardiac sarcoma. The Journal of thoracic and cardiovascular surgery 2002. link 76 Uberfuhr P, Meiser B, Fuchs A, Schulze C, Reichenspurner H, Falk M et al.. Heart transplantation: an approach to treating primary cardiac sarcoma?. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2002. link00409-6) 77 Alhadab T, Alvarez F, Phillips NJ, Hauptman PJ. Malignant fibrous histiocytoma of the lung presenting as bronchial obstruction in a heart transplant recipient. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2002. link00416-3) 78 Grubitzsch H, Wollert HG, Eckel L. Sarcoma associated with silver coated mechanical heart valve prosthesis. The Annals of thoracic surgery 2001. link02578-4) 79 Stoica SC, Mitchell IM, Foreman J, Hunt CJ, Wallwork J, Large SR. Atrial transplantation for recurrent cardiac sarcoma. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2001. link00306-0) 80 Gaumann A, Tews DS, Mayer E, Dahm M, Petrow PK, Otto M et al.. Expression of apoptosis-related proteins, p53, and DNA fragmentation in sarcomas of the pulmonary artery. Cancer 2001. link92:5<1237::aid-cncr1443>3.0.co;2-e) 81 McElhinney DB, Carpentieri DF, Bridges ND, Clark BJ, Gaynor JW, Spray TL. Sarcoma of the mitral valve causing coronary arterial occlusion in children. Cardiology in the young 2001. link 82 Gaumann A, Petrow P, Mentzel T, Mayer E, Dahm M, Otto M et al.. Osteopontin expression in primary sarcomas of the pulmonary artery. Virchows Archiv : an international journal of pathology 2001. link 83 Nishida N, Yutani C, Ishibashi-Ueda H, Tsukamoto Y, Ikeda Y, Nakamura Y. Histopathological characterization of aortic intimal sarcoma with multiple tumor emboli. Pathology international 2000. link 84 Simpson WL, Mendelson DS. Pulmonary artery and aortic sarcomas: cross-sectional imaging. Journal of thoracic imaging 2000. link 85 Rückert RI, Kröncke TJ, Bürger K. Long-term survival after radical resection of a primary angiosarcoma of the innominate vein. The Annals of thoracic surgery 2000. link01961-5) 86 Reising C, Thieman K, Nuss F, Latcham AP. Primary cardiac angiosarcoma with right coronary artery-to-pericardial fistula--a case report. Angiology 1999. link 87 Ceresoli G, Passoni P, Benussi S, Alfieri O, Dell'Antonio G, Bolognesi A. Primary cardiac sarcoma in pregnancy: a case report and review of the literature. American journal of clinical oncology 1999. link 88 Weijmer MC, Kummer JA, Thijs LG. Case report of a patient with an intimal sarcoma of the pulmonary trunk presenting as a pulmonary embolism. The Netherlands journal of medicine 1999. link00056-x) 89 Movsas B, Teruya-Feldstein J, Smith J, Glatstein E, Epstein AH. Primary cardiac sarcoma: a novel treatment approach. Chest 1998. link 90 Llombart-Cussac A, Pivot X, Contesso G, Rhor-Alvarado A, Delord JP, Spielmann M et al.. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. British journal of cancer 1998. link 91 Ingeholm P, Engel P. Metastasizing sarcoma of the aorta. A case report. APMIS : acta pathologica, microbiologica, et immunologica Scandinavica 1997. link 92 Savoia MT, Liguori C, Nahar T, Marboe C, Weinberger J, Di Tullio MR et al.. Transesophageal echocardiography-guided transvenous biopsy of a cardiac sarcoma. 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Primary cardiac malignancy presenting as left atrial myxoma. Clinical and surgical considerations. Minerva chirurgica 1996. link 98 Nanjo H, Murakami M, Ebina T, Hoshi N, Sasaki T, Zhuang YJ et al.. Aortic intimal sarcoma with acute myocardial infarction. Pathology international 1996. link 99 Shijubo N, Nakata H, Sugaya F, Imada A, Suzuki A, Kudoh K et al.. Malignant hemangioendothelioma of the aorta. Internal medicine (Tokyo, Japan) 1995. link 100 al-Robaish A, Lien DC, Slatnik J, Nguyen GK. Sarcoma of the pulmonary artery trunk: report of a case complicated with hemopericardium and cardiac tamponade. The Canadian journal of cardiology 1995. link

    Original source

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